Abstract
Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a necrotizing vasculitis affecting small blood vessels in a trio of anatomic locations—the upper airways, lower respiratory tract, and kidneys. Serologically, GPA typically shows cytoplasmic-antineutrophil cytoplasmic antibodies (c-ANCA) positivity. Radiographic studies and gross examination of the lungs will often display bilateral nodules. The microscopic hallmark of GPA is a trio of vasculitis, parenchymal necrosis, and granulomatous inflammation. Given the findings of granulomas and necrosis, the differential diagnosis and etiologic considerations include other granulomatous processes such as primary and secondary vasculitis, infection, drug toxicity, and collagen-vascular disease.
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Lanham J, Elkon K, Pusey C, Hughes G. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine. 1984;63(2):65–81.
Mark E, Flieder D, Matsubara O. Treated Wegener’s granulomatosis: distinctive pathological findings in the lungs of 20 patients and what they tell us about the natural history of the disease. Hum Pathol. 1997;28(4):450–8.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11.
Travis WD, Colby TV, Lombard C, Carpenter HA. A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation. Am J Surg Pathol. 1990;14(12):1112–25.
Mahr AD. Epidemiological features of Wegener’s granulomatosis and microscopic polyangiitis: two diseases or one “anti-neutrophil cytoplasm antibodies-associated vasculitis” entity? APMIS Suppl. 2009;117(s127):41–7.
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997;337(21):1512–23.
Falk RJ, Hogan S, Carey TS, Jennette JC. Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network. Ann Intern Med. 1990;113(9):656–63.
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488–98.
Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98(1):76–85.
Roa JK, Allen NB, Pincus T. Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis. Ann Intern Med. 1998;129(5):345–52.
Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66(2):222–7.
Rao DA, Wei K, Merola JF, O’Brien WR, Takvorian SU, Dellaripa PF, et al. Myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) and proteinase 3-ANCA without immunofluorescent ANCA found by routine clinical testing. J Rheumatol. 2015;42(5):847–52.
Kallenberg CG. Key advances in the clinical approach to ANCA-associated vasculitis. Nat Rev Rheumatol. 2014;10(8):484–93.
Hagen EC, Daha MR, Hermans J, Andrassy K, Csernok E, Gaskin G, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization. Kidney Int. 1998;53(3):743–53.
Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest. 1990;97(4):906–12.
Bomanji JB, Gupta N, Gulati P, Das CJ. Imaging in tuberculosis. Cold Spring Harb Perspect Med. 2015;5(6):5.
Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener’s granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol. 1991;15(4):315–33.
Lombard CM, Duncan SR, Rizk NW, Colby TV. The diagnosis of Wegener’s granulomatosis from transbronchial biopsy specimens. Hum Pathol. 1990;21(8):838–42.
Uner A, Rozum-Slota B, Katzenstein A. Bronchiolitis obliterans-organizing pneumonia (BOOP)-like variant of Wegener’s granulomatosis: a clinicopathologic study of 16 cases. Am J Surg Pathol. 1996;20(7):794–801.
Travis WD. Common and uncommon manifestations of Wegener’s granulomatosis. Cardiovasc Pathol. 1994;3(3):217–25.
Gaurner J, Brandt ME. Histopathologic diagnosis of fungal infections in the 21st century. Clin Microbiol Rev. 2011;24(2):247–80.
Kommareddi S, Abramowsky C, Swinehart G, Hrabak L. Nontuberculous mycobacterial infections: comparison of the fluorescent auramine-O and Ziehl-Neelsen techniques in tissue diagnosis. Hum Pathol. 1984;15(11):1085–9.
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Scapa, J.V., Fishbein, G.A. (2022). Granulomatosis with Polyangiitis Versus Mycobacterial/Fungal Infection. In: Xu, H., Ricciotti, R.W., Mantilla, J.G. (eds) Practical Lung Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-031-14402-8_49
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